2018 Volume 35 Issue 2 Pages 122-126
A 45–year–old woman presented generalized convulsion followed by getting restlessness and less speech. Fluid–attenuated inversion recovery (FLAIR) images of brain MRI showed high intensity lesion in bilateral amygdara and hippocampus. Serum antibody screening showed positivity for anti–leucine–rich glioma inactivated–1 (LGI–1) antibody. We diagnosed her anti–LGI1 limbic encephalitis. She received both methylprednisolone pulse therapy and plasmapheresis and showed remarkable improvement of clinical symptoms and MRI abnormality. However, one month later, she showed recurrence with psychological symptom. After methylprednisolone pulse therapy, we started oral prednisolone for relapse prevention. About two years later, she presented left upper limb chorea, and FLAIR image of brain MRI revealed high intensity area in right caudate nucleus, putamen and pallidum. After methylprednisolone pulse therapy, we added azathioprine for relapse prevention. From the point of inhibition of cognitive impairment, we should consider aggressive immunotherapy, including oral prednisolone and immunosuppressant for anti–LGI–1 limbic encephalitis.